Affordable Access

Podocyte differentiation and hereditary proteinuria/nephrotic syndromes.

Authors
Type
Published Article
Journal
Journal of the American Society of Nephrology : JASN
Publication Date
Volume
14 Suppl 1
Identifiers
PMID: 12761234
Source
Medline
License
Unknown

Abstract

The study of familial nephrotic syndromes (NS) and the analysis of murine models of glomerular diseases resulted in major progresses in the knowledge of podocyte physiology and pathology. Numerous proteins participating in the composition of the slit diaphragm region have been identified. The importance of several of them (nephrin, podocin, CD2AP, and Neph1) in the maintenance of the glomerular filtration barrier has been demonstrated by the occurrence of massive proteinuria when they are defective. The role of the cytoskeleton has been revealed by the development of proteinuria/NS in patients with ACTN4 mutation and the occurrence of early and severe NS in alpha-actinin-4-deficient mice. Given the genetic heterogeneity of familial NS and the many other genes to be identified, further insights in the molecular basis of the role of the podocyte in the maintenance of the glomerular filtration barrier may be expected in the near future.

Statistics

Seen <100 times